Department of Cellular Pathology, Hospital Universitario El Bierzo, Ponferrada, Spain.
Department of Cellular Pathology, Hospital de la Reina, Ponferrada, Spain.
J Cutan Pathol. 2020 Aug;47(8):729-733. doi: 10.1111/cup.13684. Epub 2020 Apr 1.
Classic Ehlers-Danlos syndrome (EDS) is a connective tissue disorder characterized by laxity. The skin, as one of the organs involved, shows hyperextensibility, which makes it prone to trauma. In this context, it would seem logical for cutaneous synovial metaplasia, which is considered a form of repair, to be commonly found in cases of EDS. However, there are only two previously published cases of synovial metaplasia in EDS. We present a third case in a 56-year-old woman with painful redundant skin in both elbows and knees for whom a skin fold of the left elbow was removed to relieve her symptoms. The biopsy showed preservation of the elastic and collagen fibers. The main alteration was the evidence of dermal cystic spaces lined by fibrinoid rests with focal pseudopapillary projections. However, in some zones the cellular lining was preserved, and it was composed of vimentin-positive, fibroblast-like flat, elongated cells, as well as CD68-positive macrophages. No birefringent particles were found in an examination under polarized light.
经典型埃勒斯-当洛斯综合征（EDS）是一种结缔组织疾病，其特征为松弛。皮肤作为受累器官之一，表现为过度伸展性，使其容易受到创伤。在这种情况下，皮肤滑膜化生（被认为是一种修复形式）在 EDS 病例中常见似乎合乎逻辑。然而，此前仅发表过两例 EDS 中的滑膜化生病例。我们报告了第三例病例，患者为 56 岁女性，双肘和双膝处有疼痛性多余皮肤，为缓解症状切除了左肘的皮肤褶皱。活检显示弹性纤维和胶原纤维保留。主要改变为真皮囊腔的证据，由纤维蛋白样残留物排列，伴有局灶性假乳头状突起。然而，在一些区域保留了细胞衬里，由波形蛋白阳性、成纤维细胞样的扁平、伸长细胞以及 CD68 阳性巨噬细胞组成。偏振光检查未发现双折射颗粒。
